BACKGROUND AND PURPOSE: Intracranial haemorrhage in neurosarcoidosis (NS-ICH) is rare, poorly understood and the diagnosis of NS may not be immediately apparent. METHODS: The clinical features of three new NS-ICH cases are described including new neuropathological findings and collated with cases from a systematic literature review. RESULTS: Cases: (i) A 41-year-old man with headaches, hypoandrogenism and encephalopathy developed a cerebellar haemorrhage. He had neuropathological confirmation of NS with biopsy-proven angiocentric granulomata and venous disruption. He responded to immunosuppressive therapy. (ii) A 41-year-old man with no history of hypertension was found unconscious. A subsequently fatal pontine haemorrhage was diagnosed. Liver biopsy revealed sarcoid granulomas. (iii) A 36-year-old man with raised intracranial pressure headaches presented with a seizure and a frontal haemorrhage. Hilar lymph node biopsy confirmed sarcoidosis, and he was treated successfully. Systematic Review: Twelve other published cases were identified and collated with our cases. Average age was 36 years and M:F = 2.3:1; 46% presented with neurological symptoms and 31% had CNS-isolated disease. Immediate symptoms of ICH were acute/worsening headache or seizures (60%). ICH was supratentorial (62%), infratentorial (31%) or subarachnoid (7%). 40% had definite NS, 53% probable NS and 7% possible NS (Zajicek criteria). Antigranulomatous/immunosuppressive therapy regimens varied and 31% died. CONCLUSIONS: This series expands our knowledge of the pathology of NS-ICH, which may be of arterial or venous origin. One-third have isolated NS. Clinicians should consider NS in young-onset ICH because early aggressive antigranulomatous therapy may improve outcome.

Item Type:

Article

Language:

en

ISSN:

1468-1331

Full metadata record

DC Field

Value

Language

dc.contributor.author

O'Dwyer, J P

en_GB

dc.contributor.author

Al-Moyeed, B A

en_GB

dc.contributor.author

Farrell, M A

en_GB

dc.contributor.author

Pidgeon, C N

en_GB

dc.contributor.author

Collins, D R

en_GB

dc.contributor.author

Fahy, A

en_GB

dc.contributor.author

Gibney, J

en_GB

dc.contributor.author

Swan, N

en_GB

dc.contributor.author

Dempsey, O J

en_GB

dc.contributor.author

Kidd, D P

en_GB

dc.contributor.author

Reid, J M

en_GB

dc.contributor.author

Smyth, S

en_GB

dc.contributor.author

McCabe, D J H

en_GB

dc.date.accessioned

2012-12-05T12:36:26Z

-

dc.date.available

2012-12-05T12:36:26Z

-

dc.date.issued

2012-06-09

-

dc.identifier.citation

Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature. 2012:notEur J Neurol

en_GB

dc.identifier.issn

1468-1331

-

dc.identifier.pmid

22681045

-

dc.identifier.doi

10.1111/j.1468-1331.2012.03783.x

-

dc.identifier.uri

http://hdl.handle.net/10147/254547

-

dc.description.abstract

BACKGROUND AND PURPOSE: Intracranial haemorrhage in neurosarcoidosis (NS-ICH) is rare, poorly understood and the diagnosis of NS may not be immediately apparent. METHODS: The clinical features of three new NS-ICH cases are described including new neuropathological findings and collated with cases from a systematic literature review. RESULTS: Cases: (i) A 41-year-old man with headaches, hypoandrogenism and encephalopathy developed a cerebellar haemorrhage. He had neuropathological confirmation of NS with biopsy-proven angiocentric granulomata and venous disruption. He responded to immunosuppressive therapy. (ii) A 41-year-old man with no history of hypertension was found unconscious. A subsequently fatal pontine haemorrhage was diagnosed. Liver biopsy revealed sarcoid granulomas. (iii) A 36-year-old man with raised intracranial pressure headaches presented with a seizure and a frontal haemorrhage. Hilar lymph node biopsy confirmed sarcoidosis, and he was treated successfully. Systematic Review: Twelve other published cases were identified and collated with our cases. Average age was 36 years and M:F = 2.3:1; 46% presented with neurological symptoms and 31% had CNS-isolated disease. Immediate symptoms of ICH were acute/worsening headache or seizures (60%). ICH was supratentorial (62%), infratentorial (31%) or subarachnoid (7%). 40% had definite NS, 53% probable NS and 7% possible NS (Zajicek criteria). Antigranulomatous/immunosuppressive therapy regimens varied and 31% died. CONCLUSIONS: This series expands our knowledge of the pathology of NS-ICH, which may be of arterial or venous origin. One-third have isolated NS. Clinicians should consider NS in young-onset ICH because early aggressive antigranulomatous therapy may improve outcome.

en_GB

dc.language

ENG

-

dc.language.iso

en

en

dc.rights

Archived with thanks to European journal of neurology : the official journal of the European Federation of Neurological Societies

en_GB

dc.title

Neurosarcoidosis-related intracranial haemorrhage: three new cases and a systematic review of the literature.